Primary pure large cell neuroendocrine carcinoma of the ovary: A rare case report and review of literature

Abstract

Introduction: Ovarian large cell neuroendocrine carcinoma (LCNEC), or ovarian non-small cell neuroendocrine carcinoma, which is a newly described tumour in the classification of primary ovarian neoplasms by the World Health Organization, is a rare entity that is frequently associated with a surface epithelial and germ cell neoplasm component. Few cases have been reported in the literature, and only 18 primary pure ovarian LCNEC cases have been reported so far, including our 1 case. Ovarian LCNEC is a highly aggressive tumor with a poor prognosis even at an early stage.

Patient concerns: We report a case of a 55-year-old postmenopausal woman who complained of abdominal pain. CT examination revealed a mass in the right adnexial region and CA125 level was elevated.

Diagnosis: She underwent a exploratory laparotomy, and diagnosed as LCNEC histopathologically.

Interventions: Cytoreductive surgery was administered to the patient, and had accepted 5 cycles of chemotherapy consisting of paclitaxel and cisplatin.

Outcomes: Follow-up for 12 months showed no clinical or radiological evidence of disease recurrence.

Conclusion: This case is 1 of the ovarian LCNEC which is a rare and extremely malignant tumor. Diagnosis requires histopathology and immunohistochemistry. The treatment includes primary cytoreductive surgery followed by chemotherapy.